Lichen Planus of the Vulva

Vulvar lichen planus (LP) is a chronic, T-cell mediated inflammatory mucocutaneous disorder affecting the vulva and often the vagina, with scarring and architectural destruction. It is probably under recognised, and prevalence is not well established. The mean age of onset is 50 to 60 years.

It may be isolated or occur with oral LP and other mucosal and cutaneous sites (vulvovaginal gingival syndrome).

Subtypes:

• erosive: the commonest and most symptomatic, with chronic erosions on the vestibule and labia minora
• papulosquamous: classic violaceous flat topped papules and lacy Wickham striae
• hypertrophic

Associated conditions: hepatitis C, other autoimmune disease (thyroid, vitiligo, alopecia areata), and drugs (lichenoid drug eruptions). Risk of vulvar squamous cell carcinoma is increased (about 1 to 3%, mainly in erosive disease).

• Vulvar burning, soreness, pain, pruritus, dyspareunia, and post-coital bleeding; copious purulent vaginal discharge in desquamative inflammatory vaginitis
• Examination: well demarcated glazed, bright red erosions at the vestibule and inner labia minora with a white reticulate border (Wickham striae), loss of labia minora, clitoral hood phimosis and burying of the clitoris, vestibular scarring and narrowing of the introitus
• Vaginal involvement: synechiae, agglutination, stenosis, adhesions, and obliteration of the vaginal vault
• Extragenital: oral (reticular, erosive gingivitis), skin (violaceous papules on wrists, shins), nails, scalp (lichen planopilaris), oesophagus (dysphagia, strictures)

• Clinical features supported by biopsy from the edge of an erosion or a non-eroded area (4 mm punch).
• Histology: band like lymphocytic infiltrate at the dermo epidermal junction, basal cell liquefaction, saw tooth rete ridges, Civatte bodies.
• Direct immunofluorescence (shaggy fibrinogen deposition) to exclude pemphigoid and pemphigus vulgaris.
• Vaginal swabs and wet mount for infection (candida, bacterial vaginosis, trichomonas), pH, and exclusion of desquamative inflammatory vaginitis
• Hepatitis C serology and liver function tests; thyroid tests
• Examination of mouth, skin, nails, scalp, and oesophageal assessment if dysphagia
• Review of medications (beta blockers, ACE inhibitors, thiazides, NSAIDs, antimalarials)
• Re-biopsy of non-healing ulcers or indurated areas for squamous cell carcinoma and differentiated vulvar intraepithelial neoplasia

• General: gentle skincare, soap substitutes, emollients, avoid irritants.
• First line: ultrapotent topical corticosteroid. Clobetasol propionate 0.05% ointment once daily to the affected skin for 4 to 12 weeks, then taper to 2 to 3 times weekly or as needed for maintenance.
• For vaginal disease, use hydrocortisone acetate 25 to 100 mg suppositories or hydrocortisone 10% foam intravaginally nightly then tapering, or clobetasol 0.05% via applicator. Vaginal steroid is key for preventing synechiae.
• Topical calcineurin inhibitors (tacrolimus 0.1% ointment twice daily, pimecrolimus 1% cream) for steroid refractory disease or steroid sparing (with a small theoretical cancer concern).
• Intralesional triamcinolone 10 mg/mL for localised refractory areas.
• Systemic therapy for severe or refractory disease: prednisolone 0.5 to 1 mg/kg/day for 2 to 4 weeks (short course for flares), methotrexate 7.5 to 15 mg weekly with folic acid 5 mg weekly, mycophenolate mofetil 1 to 2 g daily, hydroxychloroquine 200 to 400 mg daily, azathioprine 1 to 2 mg/kg/day, ciclosporin 3 to 5 mg/kg/day.
• Acitretin 25 mg daily for hypertrophic LP. In resistant cases, biologics (adalimumab, secukinumab) or JAK inhibitors.
• Prevent and treat scarring: regular vaginal dilation (graduated dilators, with topical steroid) to prevent synechiae; surgical lysis of adhesions and dilation of stenosis once inflammation is controlled; oestrogen cream for atrophy.
• Pain: lidocaine 5% ointment, gabapentin or amitriptyline for neuropathic pain.
• Treat secondary infection (candidiasis) and consider treatment of hepatitis C.
• Long term surveillance: every 6 to 12 months (more frequently initially) for relapse and early detection of squamous cell carcinoma and vulvar intraepithelial neoplasia, with a low threshold for biopsy.